Gene Symbol: KCNG3
Description: potassium voltage-gated channel modifier subfamily G member 3
Alias: KV10.1, KV6.3, potassium voltage-gated channel subfamily G member 3, potassium channel, voltage gated modifier subfamily G, member 3, potassium voltage-gated channel, subfamily G, member 3, voltage-gated potassium channel 6.3, voltage-gated potassium channel Kv10.1, voltage-gated potassium channel subunit Kv10.1, voltage-gated potassium channel subunit Kv6.3, voltage-gated potassium channel subunit Kv6.4
Species: human
Products:     KCNG3

Top Publications

  1. Pardo L, Gomez Varela D, Major F, Sansuk K, Leurs R, Downie B, et al. Approaches targeting K(V)10.1 open a novel window for cancer diagnosis and therapy. Curr Med Chem. 2012;19:675-82 pubmed
    ..1 opens a novel window for treating cancer. In this review we will give an overview of the current status of data linking K(V)10.1 to cancer, and propose techniques that could exploit K(V)10.1's properties for the management of cancer. ..
  2. Bani Fatemi A, Zai C, De Luca V. Early onset schizophrenia: Gender analysis of genome-wide potential methylation. Clin Chim Acta. 2015;449:63-7 pubmed publisher
    ..000008). In the gene-wise analysis, the KCNG3 was significantly associated with higher potential methylation in males (p=0.0004)...
  3. Kortüm F, Caputo V, Bauer C, Stella L, Ciolfi A, Alawi M, et al. Mutations in KCNH1 and ATP6V1B2 cause Zimmermann-Laband syndrome. Nat Genet. 2015;47:661-7 pubmed publisher
    ..Structural analysis predicts a perturbing effect of the mutation on complex assembly. Our findings demonstrate that KCNH1 mutations cause ZLS and document genetic heterogeneity for this disorder. ..
  4. Ufartes R, Schneider T, Mortensen L, de Juan Romero C, Hentrich K, Knoetgen H, et al. Behavioural and functional characterization of Kv10.1 (Eag1) knockout mice. Hum Mol Genet. 2013;22:2247-62 pubmed publisher
    ..1 does not show a marked phenotype is a prerequisite for utilizing Kv10.1 blocking and/or reduction techniques, such as siRNA, to treat cancer. ..
  5. Simons C, Rash L, Crawford J, Ma L, Cristofori Armstrong B, Miller D, et al. Mutations in the voltage-gated potassium channel gene KCNH1 cause Temple-Baraitser syndrome and epilepsy. Nat Genet. 2015;47:73-7 pubmed publisher
    ..Consistent with recent reports, this finding demonstrates that the etiology of many unresolved CNS disorders, including epilepsies, might be explained by pathogenic mosaic mutations. ..
  6. Urrego D, Sánchez A, Tomczak A, Pardo L. The electric fence to cell-cycle progression: Do local changes in membrane potential facilitate disassembly of the primary cilium?: Timely and localized expression of a potassium channel may set the conditions that allow retraction of the primary cil. Bioessays. 2017;39: pubmed publisher
    ..This could explain the influence of Kv10.1 in cell proliferation, as well as phenotypic features of patients carrying gain of function mutations in the gene. ..
  7. Tomczak A, Fernández Trillo J, Bharill S, Papp F, Panyi G, Stuhmer W, et al. A new mechanism of voltage-dependent gating exposed by KV10.1 channels interrupted between voltage sensor and pore. J Gen Physiol. 2017;149:577-593 pubmed publisher
  8. Hartung F, Pardo L. Guiding TRAIL to cancer cells through Kv10.1 potassium channel overcomes resistance to doxorubicin. Eur Biophys J. 2016;45:709-719 pubmed
    ..In conclusion, we propose a novel strategy to overcome resistance to chemotherapy in cancer cells. Doxorubicin and scFv62-TRAIL reciprocally sensitize the cells to each other, specifically in Kv10.1-positive tumor cells. ..
  9. Fukai R, Saitsu H, Tsurusaki Y, Sakai Y, Haginoya K, Takahashi K, et al. De novo KCNH1 mutations in four patients with syndromic developmental delay, hypotonia and seizures. J Hum Genet. 2016;61:381-7 pubmed publisher
    ..G496E may also disrupt the proper function of the Kv channel pore. Our report confirms that KCNH1 mutations are associated with syndromic neurodevelopmental disorder, and also support the functional importance of the S4 domain. ..

More Information


  1. Regnier G, Bocksteins E, Van de Vijver G, Snyders D, Van Bogaert P. The contribution of Kv2.2-mediated currents decreases during the postnatal development of mouse dorsal root ganglion neurons. Physiol Rep. 2016;4: pubmed publisher
    ..In addition, we observed age-dependent fluctuations in themRNAlevels of the Kv6.3, Kv8.1, Kv9.1, and Kv9.3 subunits. These results support an important role of both Kv2 and KvS subunits in the postnatal maturation ofDRGneurons. ..
  2. Urrego D, Movsisyan N, Ufartes R, Pardo L. Periodic expression of Kv10.1 driven by pRb/E2F1 contributes to G2/M progression of cancer and non-transformed cells. Cell Cycle. 2016;15:799-811 pubmed publisher
    ..Our results strongly suggest that Kv10.1 expression is coupled to cell cycle progression and facilitates G2/M progression in both healthy and tumor cells. ..
  3. Horst C, Titze de Almeida R, Titze de Almeida S. The involvement of Eag1 potassium channels and miR-34a in rotenone-induced death of dopaminergic SH-SY5Y cells. Mol Med Rep. 2017;15:1479-1488 pubmed publisher
    ..The neuroprotective effect of mir?34a inhibitors merits further investigations in animal models of Parkinson's disease. ..
  4. Li Z, Zhu K, Gong X, Vasilescu S, Sun Y, Hong K, et al. Inducing Polyclonal Eag1-Specific Antibodies by Vaccination with a Linear Epitope Immunogen and Its Relation to Breast Tumorigenesis. Pathol Oncol Res. 2017;23:761-767 pubmed publisher
    ..These results also suggest that Eag1 gene is a putative growth-promoting gene that might be involved in breast tumorigenesis and development. Eag1 might further be represented as a potential target for some human diseases treatment. ..
  5. Sánchez A, Urrego D, Pardo L. Cyclic expression of the voltage-gated potassium channel KV10.1 promotes disassembly of the primary cilium. EMBO Rep. 2016;17:708-23 pubmed publisher
    ..Thus, modulation of ciliogenesis by KV10.1 can explain the influence of KV10.1 expression on the proliferation of normal cells and is likely to be a major mechanism underlying its tumorigenic effects. ..
  6. Ouadid Ahidouch H, Ahidouch A, Pardo L. Kv10.1 K(+) channel: from physiology to cancer. Pflugers Arch. 2016;468:751-62 pubmed publisher
    ..The possible mechanisms by which Kv10.1 channel affects tumor cell migration and survival in breast cancer and its regulation by extracellular proteins are discussed. ..
  7. Gomez Lagunas F, Carrillo E, Pardo L, Stuhmer W. Gating Modulation of the Tumor-Related Kv10.1 Channel by Mibefradil. J Cell Physiol. 2017;232:2019-2032 pubmed publisher
    ..1 channels, thereby modifying the gating of the channels in a way that in some, but not all, aspects opposes to the gating effects exerted by divalent cations. J. Cell. Physiol. 232: 2019-2032, 2017. © 2016 Wiley Periodicals, Inc. ..
  8. Cázares Ordoñez V, Pardo L. Kv10.1 potassium channel: from the brain to the tumors. Biochem Cell Biol. 2017;95:531-536 pubmed publisher
    ..1 expression have shown interesting mechanistic insights about Kv10.1 role in oncogenesis, increasing the importance of identifying the cellular factors that regulate Kv10.1 expression in tumors. ..
  9. Napp J, Pardo L, Hartung F, Tietze L, Stuhmer W, Alves F. In vivo imaging of tumour xenografts with an antibody targeting the potassium channel Kv10.1. Eur Biophys J. 2016;45:721-733 pubmed
    ..In summary, we could show that the anti-Kv10.1 antibody is a promising tool for the development of novel concepts of targeted cancer therapy. ..
  10. Wu W, Gardner A, Sachse F, Sanguinetti M. Ginsenoside Rg3, a Gating Modifier of EAG Family K+ Channels. Mol Pharmacol. 2016;90:469-82 pubmed publisher
    ..Understanding the mechanism underlying the action of Rg3 may facilitate the development of more potent and selective EAG family channel activators as therapies for cardiovascular and neural disorders. ..
  11. Hsu P, Ma Y, Fang Y, Huang J, Gan Y, Chang P, et al. Cullin 7 mediates proteasomal and lysosomal degradations of rat Eag1 potassium channels. Sci Rep. 2017;7:40825 pubmed publisher
    ..Together, these results indicate that Cul7 mediates both proteasomal and lysosomal degradations of rEag1. Our findings provide a novel insight to the mechanisms underlying ER and peripheral protein quality controls of Eag1 channels. ..
  12. Ramos Gomes F, Romaniello V, Sánchez A, Weber C, Narayanan P, Psol M, et al. Alternatively Spliced Isoforms of KV10.1 Potassium Channels Modulate Channel Properties and Can Activate Cyclin-dependent Kinase in Xenopus Oocytes. J Biol Chem. 2015;290:30351-65 pubmed publisher
    ..Our observations highlight the relevance of noncanonical functions for the oncogenicity of KV10.1, which need to be considered when ion channels are targeted for cancer therapy. ..
  13. Moreels L, Peigneur S, Yamaguchi Y, Vriens K, Waelkens E, Zhu S, et al. Expanding the pharmacological profile of ?-hefutoxin 1 and analogues: A focus on the inhibitory effect on the oncogenic channel Kv10.1. Peptides. 2017;98:43-50 pubmed publisher
    ..Hefutoxin 1 showed low activity against the plant pathogen Fusarium culmorum and no activity against three other yeast and fungal species, even at high concentrations (?100?M). ..
  14. Barriga Montoya C, Huanosta Gutiérrez A, Reyes Vaca A, Hernández Cruz A, Picones A, Gómez Lagunas F. Inhibition of the K+ conductance and Cole-Moore shift of the oncogenic Kv10.1 channel by amiodarone. Pflugers Arch. 2018;470:491-503 pubmed publisher
    ..Our observations are interpreted considering the structural-functional characteristics of these channels. We conclude that amiodarone possibly binds with high affinity to the voltage sensor module, altering the gating of Kv10.1. ..
  15. Vega Saenz de Miera E. Modification of Kv2.1 K+ currents by the silent Kv10 subunits. Brain Res Mol Brain Res. 2004;123:91-103 pubmed
    ..The gene that encodes for Kv10.1 mRNAs maps to chromosome 2p22.1 in human, 6q12 in rat and 17E4 in mouse, locations consistent with the known systeny for human, rat and mouse chromosomes. ..
  16. Sano Y, Mochizuki S, Miyake A, Kitada C, Inamura K, Yokoi H, et al. Molecular cloning and characterization of Kv6.3, a novel modulatory subunit for voltage-gated K(+) channel Kv2.1. FEBS Lett. 2002;512:230-4 pubmed
    ..1 channels. Immunoprecipitation studies indicated that Kv6.3 bound with Kv2.1 in co-transfected cells. These results indicate that Kv6.3 is a novel member of the voltage-gated K(+) channel which functions as a modulatory subunit. ..
  17. Moreels L, Bhat C, Voráčová M, Peigneur S, Goovaerts H, Mäki Lohiluoma E, et al. Synthesis of novel purpurealidin analogs and evaluation of their effect on the cancer-relevant potassium channel KV10.1. PLoS ONE. 2017;12:e0188811 pubmed publisher
    ..1 inhibitors can be interesting anticancer drug lead compounds, the effect of 5 was evaluated on cancerous and non-cancerous cell lines. Compound 5 showed to be cytotoxic and appeared to induce apoptosis in all the evaluated cell lines...
  18. Ottschytsch N, Raes A, Van Hoorick D, Snyders D. Obligatory heterotetramerization of three previously uncharacterized Kv channel alpha-subunits identified in the human genome. Proc Natl Acad Sci U S A. 2002;99:7986-91 pubmed
    ..Including the subunits described here, the "silent subunits" represent one-third of all Kv subunits, suggesting that obligatory heterotetramer formation is more widespread than previously thought. ..
  19. Martínez R, Stühmer W, Martin S, Schell J, Reichmann A, Rohde V, et al. Analysis of the expression of Kv10.1 potassium channel in patients with brain metastases and glioblastoma multiforme: impact on survival. BMC Cancer. 2015;15:839 pubmed publisher
    ..1 expression to patients with brain metastases or GBMs and, moreover, they strongly suggest a role of tricyclic antidepressants for personalized therapy of brain malignancies. ..
  20. Moreels L, Peigneur S, Galan D, De Pauw E, Béress L, Waelkens E, et al. APETx4, a Novel Sea Anemone Toxin and a Modulator of the Cancer-Relevant Potassium Channel KV10.1. Mar Drugs. 2017;15: pubmed publisher
    ..This newly identified KV10.1 inhibitor can be used as a tool to further characterize the oncogenic channel KV10.1 or as a scaffold for the design and synthesis of more potent and safer anticancer drugs. ..
  21. Mederos y Schnitzler M, Rinné S, Skrobek L, Renigunta V, Schlichthörl G, Derst C, et al. Mutation of histidine 105 in the T1 domain of the potassium channel Kv2.1 disrupts heteromerization with Kv6.3 and Kv6.4. J Biol Chem. 2009;284:4695-704 pubmed publisher
    ..1 is required for functional heteromerization with members of the Kv6 subfamily. We conclude from our findings that Kv2.1 and Kv6.x subunits have complementary T1 domains that control selective heteromerization. ..